ABOUT GLIOMATOSIS CEREBRI 

What causes gliomatosis cerebri?

There are no known factors or conditions that would make your child more or less likely to develop gliomatosis cerebri.

How is gliomatosis cerebri classified?

The World Health Organization classification scheme includes 4 grades of glioma.

• grade I (benign)
• grade II (atypical)
• grade III (anaplastic- refers to lack of structure in the cell)
• grade IV (sarcomatous- the most serious kind of tumor)

However, since only a fraction of gliomatosis cerebri tumors are biopsied, it can be difficult to conclusively assign a grade to them. That said, they usually progress like grade IV glioblastoma multiforme tumors. These are the most aggressive kind of astrocytic tumor, and they usually have the following characteristics:

• hypercellularity (increased number of cells)
• cytologic and nuclear atypia (abnormal cells and nuclei)
• mitoses (the cells reproduce rapidly)
• necrosis (the cells die quickly)
• vascular endothelial proliferation (increased growth of blood vessels)

These tumors are aggressive, and will invade normal brain tissue. They are also likely to spread outside the central nervous system to other parts of the body.

Symptoms might come on slowly and subtly, or they might appear more abruptly. Each child may experience symptoms differently, and the most common include:

• headache and lethargy
• symptoms of increased pressure within the brain, including:
  • headache (generally upon awakening in the morning)
  • vomiting
  • seizures – can occur with high-grade gliomas in the cerebrum but are less common with low-grade lesions (any abnormal tissue).
• localized symptoms – these tumors invade normal tissue as they grow, and produce symptoms based on their location. These could include:
• weakness and other motor dysfunction
• neuroendocrine (hormones affecting the nervous system) abnormalities
• changes in behavior or thought processes

The symptoms of gliomatosis cerebri may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.

Gliomatosis cerebri is most commonly diagnosed from radiologic studies. Since often the tumor appears to have no primary location and grows aggressively, biopsies are risky. However, a biopsy may be performed if a primary mass is indentified, or if your child’s symptoms and other tests do not seem typical for the condition.

Diagnostic procedures for gliomatosis cerebri may include:

• physical examination
• computerized tomography scan (also called a CT or CAT scan)- a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays. A CT scan can assess the density of tumor tissue compared to normal brain tissue, as well as establish its mass effect (the effect of an additional space occupying mass within the closed cavity of the skull on the normal brain).
• magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI provides greater anatomical detail than CT scan and does a better job of distinguishing between tumors, tumor-related swelling and normal tissue. On an MRI, gliomatosis cerebri will typically appear as a lesion that grows without distinct borders.
• magnetic resonance spectroscopy (MRS) – a test done along with an MRI. It can detect the presence of organic compounds within sample tissue that can identify the tissue as normal or tumor, and may also be able to tell if the tumor is a glial tumor or if it is of neuronal origin (originating in a neuron, instead of in a brain cell).

Specific treatment for a gliomatosis cerebri may include:

• Radiation therapy – The primary therapy for newly diagnosed gliomatosis cerebri, radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.
• Experimental chemotherapy delivered along with radiation therapy is actively being investigated as a treatment of gliomatosis cerebri. Several trials evaluating new agents (such as thalidomide and temazolamide) are either underway or have been completed. In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses.

We should know more about the results of these tests soon. To date, no chemotherapy regimen has been shown to increase survival rates.

Unfortunately, surgery is not an option in the treatment of these tumors.

As with all pediatric cancers, care should be delivered at specialized centers where multidisciplinary teams can provide not only expert diagnostics and experienced medical, surgical and radiation oncologists, but also psychosocial support, neuro-psychological testing and specialized school plans, all delivered in a child- and family-sensitive and friendly environment.

How are side effects managed?      

Your child may experience side effects from radiation. Radiation often produces inflammation, which can temporarily make symptoms and dysfunctions worse. If your child experiences inflammation, steroids may be prescribed.

Many specialized brain tumor treatment centers have now added experts in complementary or alternative medicine (CAM). These treatments, including acupuncture/acupressure, therapeutic touch, massage, herbs and dietary recommendations, can also help to control pain and side effects of therapy. Talk to your child’s physician about whether CAM might be a viable option of treatment.

The prognosis for gliomatosis cerebri remains poor.

A variety of chemotherapeutic regimens have been evaluated in the treatment of newly diagnosed high-grade gliomas

• While studies in adults have suggested that procarbazine, vincristine and CCNU (PVC) produce modest responses in grade III gliomas, this has not been demonstrated for pediatric cases or for grade IV tumors.

• Several other regimens have also produced responses, but none has improved survival.

• Increased doses of chemotherapy in the setting of autologous bone marrow transplant have also not produced notable advantage.

Children’s Brain Tumor Project at the Weill Cornell Pediatric Brain and Spine Center

The Children’s Brain Tumor Project is a research effort at the Weill Cornell Pediatric Brain and Spine Center, co-directed by Dr. Mark Souweidane and Dr. Jeffrey Greenfield. The Children’s Brain Tumor Project has a single goal: to bring hope to the hundreds of patients and families each year who confront the heartbreaking diagnoses of rare and inoperable pediatric brain tumors such as Gliomatosis Cerebri.

Coming soon..